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European Journal of Echocardiography 2008 9(2):344-345; doi:10.1093/ejechocard/jen018
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Significant obstruction of the right and left ventricular outflow tract in a patient with biventricular hypertrophic cardiomyopathy

Thomas Butz1,*, Dieter Horstkotte1, Christoph Langer1, Hermann Esdorn2, Georg Kleikamp3, Reiner Körfer3 and Lothar Faber1

1 Department of Cardiology, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Georgstrasse 11, D-32545 Bad Oeynhausen, Germany
2 Institute of Radiology, Nuclear Medicine and Molecular Imaging, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany
3 Department of Thoracic and Cardiovascular Surgery, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany

Received 18 October 2007; accepted after revision 7 November 2007.

* Corresponding author. Tel: +49 5731 971258; fax: +49 5731 972194. E-mail address: akohlstaedt{at}hdz-nrw.de


  Abstract

Echocardiography demonstrated pronounced asymmetric left ventricular (LV) hypertrophy and thickened right ventricular muscular components in a 54-year-old woman with a history of dyspnoea (NYHA III), and recurrent syncopes. Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre.

Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) provided striking images of a right ventricular outflow tract obstruction and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis. Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to right ventricular (RV) outflow was due to massive hypertrophy of the crista supraventricularis with an ‘hour-glass’ deformity. A surgical intervention with LV septal myotomy-myectomy and RV ventriculotomy was performed successfully.

Hypertrophic obstructive cardiomyopathy with significant RV and LV outflow tract obstruction is a very rare finding.

Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because mechanisms of obstruction are different in each ventricle.

Keywords: Biventricular hypertrophic cardiomyopathy; Outflow tract obstruction; Right ventricle; Left ventricle


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